Symptoms of IgAN

The Foundation for IgA Nephropathy

Symptoms of IgAN

Patients with IgAN usually exhibit a few common symptoms (not to be confused with the uremic symptoms of more advanced renal failure). These are:

Hematuria. Blood in the urine, referred to as hematuria, is often the first sign of IgAN. Blood in the urine may originate from elsewhere in the urinary tract, so, at this point, some patients may be referred to a urologist in order to rule out non-renal causes. However, the finding of red blood cell casts and dysmorphic (small deformed, misshapen, sometimes fragmented) red blood cells combined with proteinuria is suggestive of the type of hematuria caused by IgAN and other glomerular diseases, while eumorphic red blood cells (normal sized, biconcavely shaped) are suggestive of a nonglomerular origin not related to IgAN. The colour of the urine can give a clue as to the origin of the blood. For example, cola or tea-coloured urine suggests it is coming from the glomeruli (these are the filters in the kidney that are affected by glomerular diseases like IgAN. If it's bright red, especially near the end of urination, this tends to suggest that it is coming from the bladder or urethra, and not from the kidneys. However, these are just very general guidelines. There are many other reasons for blood in the urine, so it always needs to be investigated medically until the reason or source is found. There are two presentations of hematuria, depending on how much blood is being leaked into the urine:

Macroscopic hematuria. This is when the blood is visible. It is usually called macro-hematuria for short, or gross hematuria. This may be seen as dark, tea or cola-coloured urine which the patient discovers him or herself while in the midst or shortly after an upper respiratory infection (such as a cold or flu). Reddish or pink coloured urine is more suggestive of a bleeding site other than the glomeruli. Coloured urine will often cause the person to seek a doctor's advice, and this is the more dramatic way that IgAN makes its appearance. While a person's first experience with macro-hematuria can be frightening, it really only takes a tiny amount of blood to colour the urine this way. Many people with IgAN have recurring episodes of macro-hematuria every time they get an upper respiratory infection. There is no specific treatment for blood in the urine, and episodes of visible hematuria usually resolve on their own after some time. Whether it's the first time or a recurrence, doctors will usually treat the respiratory infection that gave rise to it. However, some IgAN patients never have episodes of macro-hematuria.

Microscopic hematuria. Usually shortened as micro-hematuria. This is when blood in the urine is not visible to the naked eye. Often, micro-hematuria is first detected in the doctor's office during a routine medical examination, by means of a simple dipstick which is "dipped" in a spot urine sample. It may also be reported after a urinalysis. Such patients are usually unaware that they have anything wrong with them, or they may have gone to see a doctor for another ailment, or as is often the case, for a simple insurance exam. Micro-hematuria is one of the main features of IgAN. For the majority of patients, micro-hematuria is always present.

Proteinuria. A second symptom which may or may not be present initially is protein in the urine. As glomeruli become inflamed or damaged, they begin to allow protein to spill into the urine. Protein in the urine is called "proteinuria". As with hematuria, proteinuria may vary from very mild to heavy. Proteinuria can also be detected in the doctor's office by means of a protein dipstick. If protein is found, more precise urinalysis is usually ordered. Whether proteinuria is present at the first discovery of hematuria or not, it eventually does make its appearance. You cannot see protein in the urine, although very foamy urine is usually a reliable clue that protein is present. Normally, there should be no protein in a person's urine (except trace amounts of albumin), so even trace amounts are usually cause for further investigation. Heavy proteinuria may be treated with oral corticosteroids, as it may cause other symptoms which are independent of the IgAN itself (this is what is referred to as the nephrotic syndrome).

Nephrotic syndrome. If proteinuria is heavy enough, patients will begin to show symptoms of nephrotic syndrome. Nephrotic syndrome is not a disease in itself, but a group of symptoms which are caused by the heavy loss of protein in the urine. Nephrotic syndrome may require aggressive treatment with corticosteroids or, if that fails, other drugs, until the level of proteinuria goes down. A person usually needs to lose more 3.5 grams per day of protein in the urine before this begins to happen. Patients with nephrotic range proteinuria may experience the following main symptoms.

Edema. The main symptom is edema, or swelling of the extremities and/or the abdomen. The majority of IgAN patients do not develop nephrotic syndrome, but it is fairly common in pediatric IgAN. As IgAN further progresses, the patient will start to show some of the symptoms of chronic renal failure (discussed elsewhere on this site).

Hypoalbuminemia. Low blood levels of the protein albumin.

High cholesterol. High cholesterol commonly develops when there is heavy loss of protein in the urine.

Other symptoms. In addition to the above three main symptoms that characterize nephrotic syndrome, people who have such heavy proteinuria may also feel ill in various ways, including excessive fatigue, low appetite, facial swelling, abdominal pain along with the abdominal swelling. Also, apparent food intolerances have been reported.

Hypertension. As IgAN progresses, it is common for patients to develop high blood pressure (or hypertension). It is very important to treat hypertension by whatever means are necessary, because hypertension itself greatly increases the risk of progressing to end-stage renal disease. For more information, see the Hypertension Notebook.

Fatigue. Many report fatigue, even with mild IgAN. The fatigue we are talking about is not just ordinary tiredness after a hard day's work, but extreme, almost debilitating fatigue. In the absence of anemia, it is not known why this might happen, although it may be a crossover symptom on the continuum between IgAN and HSP. It is this author's opinion that the immune system problem which causes nephropathy to happen in the kidneys may in fact affect other aspects of the body in subtle ways which are not immediately obvious. We suspect fatigue may be one of these manifestations.

Flank pain. Also referred to as loin pain. Flank pain is also often reported. It is thought to be caused by swelling and stretching of the renal capsule (which is the covering forming the outside of the kidney) during acute episodes of the disease. This flank pain is usually reported as a dull ache. A very small percentage of patients seem to develop persistent, severe attacks of flank pain. Some of these patients may be diagnosed with loin pain hematuria syndrome (LPHS) - a condition which is not exclusive to IgAN (it remains unclear if or how this is associated with IgAN).

Joint pain. May be a crossover symptom on the continuum between IgAN and HSP which a small minority of patients may experience.

Abdominal pain. This especially occurs in conjunction with an episode of macro-hematuria. The reason is unknown, but like fatigue and joint pain, it may be a crossover symptom on the continuum between IgAN and HSP. Only a minority of patients seem to have this. In some cases, it may be associated with pancreatitis, although it is not clear if this can be directly linked to IgAN. Some people with chronic renal insufficiency or with esrd (end-stage renal disease, ie. on dialysis) may develop varying degrees of a condition called gastroparesis. This basically means delayed emptying of stomach contents, and delayed movement of food through the digestive tract. This is more common in people who have diabetes, but kidney failure patients are also more prone to it than the general population. This may cause nausea, bloating, early feeling of fullness after eating even small meals, regurgitation, abdominal pain.

Weakened immune system. A great many people on support groups talk about having a suppressed immune system. While the exact cause of IgAN is not known, it is suspected that it involves a defect in immunoglobulin A (IgA) immune complexes, and some research seems to bear this out. This should not be interpreted as having a defective, weak immune system. There is little evidence that people with IgAN have an immune system that is weaker than other people's. It could be that some medications or some supplements weaken the immune system though. For example, it has been reported that fish oil in large quantities may do this, and of course, oral steroids (like prednisone) do this also, as would other types of immune system suppressing drugs such as Imuran and Cellcept. However, you should also be aware that the more advanced your chronic renal insufficiency is, the more it tends to depress the immune system to some extent.

Other symptoms. As you progress into more advanced chronic renal insufficiency (also referred to as chronic renal failure), you may start to experience some symptoms which are common to all, regardless of whether you have IgAN or any other kidney disease. Since they are not specific to IgAN, we have listed these in the Chronic Renal Insufficiency and End-stage Renal Disease sections of the website.

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